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在现代诊断时代,小儿肺部炎性肌纤维母细胞瘤的胸部多排计算机断层扫描评估。

Thoracic Multidetector Computed Tomography Evaluation of Inflammatory Myofibroblastic Tumor of the Lung in Pediatric Patients in the Era of Modern Diagnosis.

机构信息

Departments of Radiology.

Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA.

出版信息

J Thorac Imaging. 2021 Sep 1;36(5):310-317. doi: 10.1097/RTI.0000000000000589.

Abstract

PURPOSE

The purpose of this study was to investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven inflammatory myofibroblastic tumor (IMT) of the lung in children in the era of modern understanding based on refined pathologic diagnosis.

MATERIALS AND METHODS

All pediatric patients (age 18 y and above) with a known pathologic diagnosis of IMT of the lung who underwent thoracic MDCT studies from May 2008 to December 2020 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung (nodule, mass, cyst, ground-glass opacity, consolidation), pleura (pleural effusion, pneumothorax), and mediastinum and hilum (lymphadenopathy). When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), location (laterality, lobar distribution, and intraparenchymal vs. pleural-based), borders (well-circumscribed vs. ill-defined), the presence and type of associated calcification (punctate, dense, curvilinear, or flocculent), the presence of associated cavitation, contrast enhancement pattern (homogeneous, heterogenous, central, or peripheral), and other associated findings (neural foramen involvement, anomalous vessels, mass effect, and invasion of adjacent thoracic structures) were also evaluated. Interobserver agreement between 2 independent reviewers was evaluated with κ statistics.

RESULTS

In all, 12 thoracic MDCT studies from 12 individual pediatric patients (5 males [42%] and 7 females [58%]; mean age: 9.9 y; SD: 4.4 y; range: 2 to 16 y) comprised the final study population. All 12 thoracic MDCT studies (100%) were performed with intravenous contrast. The most frequent MDCT finding of IMT of the lung in children is a solitary (92%), pleural-based (83%), well-circumscribed (100%), solid (92%) mass with heterogenous contrast enhancement (100%), often with dense calcification (50%), which occurred in both lungs and all lobes with similar frequency. No pleural abnormality (pleural effusion, pneumothorax) or mediastinal abnormality (lymphadenopathy) was detected. In addition, although mass effect on adjacent thoracic structures was frequently seen (42%), no invasion, neural foramen involvement, or associated anomalous vessels was identified. There was excellent interobserver κ agreement between 2 independent reviewers for detecting abnormalities on thoracic MDCT studies (κ>0.95).

CONCLUSIONS

IMT of the lung in children typically presents as a solitary, pleural-based, well-circumscribed, solid mass with heterogenous contrast enhancement, often with dense calcification, without significant laterality or lobar preference. In addition, pleural or mediastinal abnormalities are characteristically absent. These notable MDCT attributes of IMT of the lung are an important and novel finding, with great potential to help differentiate pediatric IMT of the lung from other thoracic masses in children.

摘要

目的

本研究旨在基于精细化病理诊断,探讨在现代认识背景下经病理证实的儿童炎性肌纤维母细胞瘤(IMT)的特征性胸部多层螺旋 CT(MDCT)表现。

材料与方法

本研究纳入了 2008 年 5 月至 2020 年 12 月期间所有经胸 MDCT 检查且已知病理诊断为肺 IMT 的儿科患者(年龄≥18 岁)。两名儿科放射科医生独立评估了肺(结节、肿块、囊肿、磨玻璃影、实变)、胸膜(胸腔积液、气胸)和纵隔及肺门(淋巴结病)的胸 MDCT 表现是否存在异常。当存在肺部异常时,评估其数量、大小、成分(实性、囊性或两者兼有)、位置(侧别、叶分布、肺内与胸膜下)、边界(边界清楚与边界不清)、是否存在及类型(点状、密集、曲线状或絮状)的相关钙化、是否存在空洞、对比增强模式(均匀、不均匀、中央或外周)和其他相关表现(神经孔受累、异常血管、肿块效应和邻近胸壁结构侵犯)。两名独立观察者之间的观察者间一致性采用 κ 统计分析。

结果

本研究最终纳入了 12 名儿童的 12 项胸部 MDCT 研究(5 名男性[42%]和 7 名女性[58%];平均年龄:9.9 岁;标准差:4.4 岁;范围:2 至 16 岁)。所有 12 项胸 MDCT 研究(100%)均采用静脉内对比剂进行。儿童肺 IMT 的最常见 MDCT 表现为单发(92%)、胸膜基底部(83%)、边界清楚(100%)、实性(92%)、不均匀增强(100%)的肿块,常伴有密集钙化(50%),这些肿块发生于双肺且各叶发生率相似。未发现胸膜异常(胸腔积液、气胸)或纵隔异常(淋巴结病)。此外,尽管经常观察到邻近胸壁结构的肿块效应(42%),但未发现侵犯、神经孔受累或伴发异常血管。两名独立观察者之间在检测胸 MDCT 研究异常方面具有极好的观察者间 κ 一致性(κ>0.95)。

结论

儿童肺 IMT 通常表现为单发、胸膜基底部、边界清楚、实性肿块,增强不均匀,常伴有密集钙化,无明显侧别或叶分布偏好。此外,胸膜或纵隔异常特征性地不存在。这些肺 IMT 的显著 MDCT 特征是一个重要的新发现,对帮助鉴别儿童肺 IMT 与儿童其他胸部肿块具有重要意义。

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